RH tends to occur either sporadically or as a subset of central nervous system (CNS) hemangioblastoma [2,3,4,5,6,7,8,9,10,11] in the Von Hippel–Lindau (VHL) syndrome (OMIM #193300) [12,13,14,15], an inherited autosomal dominant disorder caused by mutations in the VHL tumor suppressor gene (VHL, OMIM#608537). The gene discussed is VHL; the disease is von Hippel-Lindau disease.